Summary:Seventeen patients who developed hepatic veno-occlusive disease (VOD) following hematopoietic stem cell transplantation were treated with recombinant tissue plasminogen activator (rtPA) with or without heparin. rtPA was started a median of 13 days post transplant (range 4-35). All patients received rtPA at a dose of 10 mg/day as a starting dose, and 12 patients also received heparin (1500 U bolus; then 100 U/kg/day as a continuous i.v. infusion). The median number of days of rtPA therapy was 2.5 (1-12). The median total serum bilirubin level was 116 mmol/l (range 63-194) at the beginning of treatment. Six patients showed a response to rtPA treatment (29%). It was observed that by day 2 of rtPA therapy, bilirubin levels in responders showed a downwards trend as compared to those in nonresponders. In all except one patient this response was observed after two doses of rtPA. Seven out of the 11 non-responders had a past history of liver dysfunction, compared with none of the responders. There were no differences between the two groups in terms of day of onset of liver dysfunction, manifestations of disease, maximum bilirubin and creatinine levels, and day of commencing treatment. No patient experienced severe hemorrhagic complications during therapy. Four responders survived for more than 100 days compared to none of the non-responders. Probability of survival was 33% at day 100. It is difficult to unequivocally establish the role of rtPA in the treatment of VOD. The importance of bilirubin levels on days 2 or 3 of therapy in predicting outcome should be established, as should the optimum dose of rtPA and optimum duration of therapy.
The effects of desiccation on the early development stages of Mazzaella laminarioides, Scytosiphon lomentaria and Lessonia nigrescens, algal species with different patterns of distribution across the intertidal zone, were examined in the laboratory. In addition, the protective effect against desiccation was evaluated using algal extracts, including those from Porphyra columbina, a macroalga tolerant to desiccation that lives in the uppermost part of the intertidal zone. Our results showed that M. laminarioides displayed the highest resistance to daily desiccation, followed by S. lomentaria, whereas L. nigrescens was the most susceptible. Spores from L. nigrescens exposed to desiccation, although being able to germinate, ceased further post-germination development. In addition, our results showed that all species exposed to extracts from desiccated P. columbina successfully completed their development and strongly suggest the occurrence of compounds with protective properties that help in attenuating the stress caused by desiccation. Finally, our results indicate that the magnitude of the effects generated by desiccation on the early algal development is related to the position of the species in the intertidal zone, and that the protective effects of P. columbina extracts reveal an exceptional metabolism of this species under desiccation stress.
Summary:generated by gene targeting: these mutant mice developed pulmonary disease characteristic of PAP. In these animal models haematopoietic development and function were not Pulmonary alveolar proteinosis (PAP) is a disease of unknown etiopathogenesis sometimes associated with disturbed. [4][5][6] When found in association with haematological dismalignant haematological disorders. The potential reversibility of the process in these cases seems to be orders, PAP has been considered a secondary form. 7 In a series of haematology patients with respiratory failure, the related to recovery from the underlying disease. GM-CSF has acquired an important, potentially pathogenic incidence of PAP was 5.3%. 8 It has been suggested that in such cases, PAP may be the result of a disease-related role and BMT presents one therapeutic option effective in certain forms of human PAP. We present the case of defect in the AM function 9 and/or due to treatment (chemotherapy, radiotherapy, steroids). 10 The most signifia 43-year-old female patient with Ph؉ CML. During pretransplantation evaluation, unexpected pulmonary cant complication in both primary and secondary cases is superinfection, particularly by opportunistic organisms. The infiltrates were noted in the chest X-ray, PAP being diagnosed on biopsy. In view of the progressive respirpreferred diagnostic method both in primary 11 and secondary 8 PAP is bronchoalveolar lavage (BAL). atory symptomatology and her CML being in accelerated phase, the patient underwent haematopoietic transSpontaneous remission of idiopathic primary PAP has occasionally been described. 12 The use of BAL as therapy plantation. She died on day ؉12 from invasive pulmonary aspergillosis before a response could be has improved the prognosis considerably. However, the potential reversibility of the process in secondary PAP is observed. Pathogenic implications in PAP and the role of haematopoietic transplantation in this disease are related to prior recovery from the haematological condition. 8 The possible part played by a deficiency of GMdiscussed. Keywords: pulmonary alveolar proteinosis; CML; PBSC CSF in the pathogenesis of human PAP opens up new therapeutic possibilities, such as BMT 13 or treatment with transplantation; aspergillosis GM-CSF. 14 We present a case of PAP diagnosed in a female with CML. The patient underwent allogeneic haematopoietic Pulmonary alveolar proteinosis (PAP) is a heterogenous transplantation with the objective of achieving recovery and group of congenital and acquired diseases. It is characsubsequent control of the respiratory symptomatology. terized by deposition of extracellular granular material consisting of a mixture of phospholipids and surfactant proteins within the alveoli and airways. In humans, a fatal form of Case report congenital PAP arises from a mutation resulting in surfactant protein-B (SP-B) deficiency. 1 Acquired forms may be A 43-year-old female was diagnosed with CML Phϩ in found in previously healthy patients, but the pathogenic chronic phase during ho...
Background The diagnosis of hepatitis B virus (HBV) infection requires HBV DNA testing and serologic testing for detection of the surface antigen (HBsAg) and the hepatitis B core antibody (anti-HBc). There is a population of patients with occult HBV infection (OBI), which is not detected by HBsAg or HBV DNA quantification in blood, despite the presence of active replication in the liver. Scope This document provides a definition of OBI and describes the diagnostic techniques currently used. It also addresses the detection of patients with risk factors and the need for screening for OBI in these patients. Summary Correct diagnosis of OBI prevents HBV reactivation and transmission. Diagnosis of OBI is based on the detection of HBV DNA in patients with undetectable HBsAg in blood. Perspectives A high number of patients with OBI may remain undiagnosed; therefore, screening for OBI in patients with factor risks is essential. For a correct diagnosis of OBI, it is necessary that new markers such as ultrasensitive HBsAg are incorporated, and a more comprehensive marker study is performed by including markers such as cccDNA.
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